This mass was a handful

This 61-year-old right-hand dominant female presents with a left hand mass. Mass (green arrows) has been present for approximately 15 years, and has been slowly enlarging. Patient states she had left side carpal tunnel release, and noted the mass a few months later. The mass is somewhat tender, and she denies numbness or tingling.

Q1 – Which is the most likely diagnosis?
(a) Tumoral calcinosis
(b) Synovial sarcoma
(c) Myositis ossificans / heterotopic bone formation
(d) Synovial chondromatosis
(e) Pigmented Villonodular Synovitis (PVNS)

Lateral Radiograph

Coronal T2 Fat Sat

 

A1 – (a) Tumoral calcinosis

 

Q2 – Regarding tumoral calcinosis, which of the following is not true?
(a) More frequent in Blacks
(b) One third familial tendency
(c) Near joints
(d) May effect skin, marrow, and teeth (dental pulp Ca+ and root enlargement)
(e) No known predisposing factors

Q3 – Regarding tumoral calcinosis, which of the following statements is false?
(a) Predisposing factors are metabolic and related to calcium, phosphorus, and vitamin D
(b) Encapsulated rather than spreading
(c) Fibrous septa
(d) Inflammatory component common
(e) “Zonule effect”

Q4 – Regarding tumoral calcinosis, which is not true regarding imaging?
(a) Prefers extensor surface
(b) Radiolucent septa “chicken wire” appearance
(c) Commonly involves intra-articular space
(d) MR/CT calcium-fluid level “sedimentation sign”
(e) High signal around low signal on T2 MR

Q5 – Regarding tumoral calcinosis, which is not true regarding imaging?
(a) Average number of lesions per patient is ~ 3
(b) Bone scan is negative
(c) “Sedimentation sign” on MR/CT = more active disease
(d) Pseudoxanthoma elasticum-like syndrome of skin, vessels and retina
(e) Associated CPPD arthrosis or calcific myelitis

 

A2 – (e) No known predisposing factors
A3 – (e) “Zonule effect”
A4 – (c) Commonly involves intra-articular space
A5 – (b) Bone scan is negative

 

Differential Diagnosis of Soft Tissue Masses in or about the Joints:

1. TUMOR-LIKE: Tumoral calcinosis, pigmented villonodular synovitis, ganglion pseudocyst, synovial cyst, myositis ossificans, heterotopic bone

2. BENIGN: Synovial lipoma, myxoma, synovial chondromatosis/chondroma, nodular fasciitis

3. MALIGNANT: Synovial sarcoma, clear cell sarcoma

Tumoral Calcinosis Clinical Features:

1. Usually children and young adults

2. Increased incidence in black / African-Americans

3. Familial tendency in 33% of the cases

4. When familial, autosomal dominant with variable expression

5. Calcified bulky periarticular mass around the hip, elbow, shoulder, foot or wrist

6. Can be associated with CPPD and pseudoxanthoma elasticum syndrome

7. Skin ulceration, marrow and dental changes

8. The etiology is most likely metabolic with an elevated calcium phosphorus product and sometimes increased vitamin D. Trauma and idiopathic causes have been reported. Some association with renal dysfunction in secondary form.

Tumoral Calcinosis Radiography:

1. Extensor surface calcified periarticular mass

2. Radiolucent septations or “chicken wire sign” as opposed to the “zonule sign” of myositis ossificans. Zonule sign = calcification / ossification from the outside to inside

3. Range of motion preserved unlike myositis ossificans

4. Extraarticular bursa created around the lesion

5. More often multifocal than myositis ossificans average three lesions per individual

6. Calcium fluid levels on CT and MR so-called “liquid calcium or sedimentation sign” unlike myositis ossificans; sedimentation sign equals more active disease

7. Hot on bone scan

MRI:

1. Usually low central signal on T1

2. Usually high signal on T2 peripherally, the opposite of myositis ossificans due to adventitial bursae. In myositis ossificans, the high signal is usually in the periphery eg “zonule phenomenon.”

3. High signal interstitial septations water-like in character

4. Pseudoxanthoma elasticum associated with skin and vascular calcification with retinal angioid streaks

5. Associated CPPD arthropathy

6. Dental abnormalities may include root enlargement and intrapulp calcification, marrow involvement as areas of calcification

Differential Diagnosis of Periarticular Calcification Includes:

1. Scleroderma

2. Other collagen vascular diseases, especially dermato-/polymyositis

3. Chronic renal failure so-called secondary tumoral calcinosis with elevated Ca+, calcium-phosphorus product

4. Milk-alkali syndrome

5. Synovial sarcoma- usually periarticular. Bulky, aggressive, heterogeneous mixed T2 intermediate signal.

6. Trauma or traumatic exostosis (including turret exostosis)

Tumoral Calcinosis Treatment:

1. Phosphate depletion therapy using:

(a) Aluminum hydroxide
(b) Acetazolamide

2. Surgical excision

 

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